Family support weighs heavily in doctors’ decisions on ventilation

Survey study of key factors that influence advanced care of teens with Rett

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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When clinicians have to decide whether to recommend invasive ventilation for adolescents with Rett syndrome, parental and familial support are the biggest factors in their decision, a recent study reports.

Many teenagers with Rett syndrome have difficulty breathing, and clinicians for some may recommend invasive ventilation via a tracheostomy — a surgical intervention that inserts a tube through the throat and into the windpipe to help with breathing.

Undergoing invasive ventilation is a major life change, and lifelong care is required to keep the equipment in good working order. As rights for people with disabilities continue to advance, there’s been increasing focus on the importance of centering patients and their families in discussions about major medical decisions like invasive ventilation.

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Vignettes of patients in varying situations presented to clinicians

“The enhancement of appropriate decision-making participation opportunities for both the child/adolescent with [Rett] and their parents, requires compassionate, empathic healthcare environments based on respect and trust, especially when the decision is as consequential as the initiation of life-sustaining technology dependence,” the researchers wrote.

But there hasn’t been much research into how these decisions are made from a clinicians’ perspective, and “this impedes transparency concerning how such decisions are made,” the scientists wrote.

To help facilitate these conversations, a research team in Ireland conducted a global survey of clinicians, aiming to identify factors that play the biggest role in influencing whether or not they recommend invasive ventilation for adolescents with Rett syndrome. The study, “Adolescents with Rett syndrome at critical care pathway junctures: Examining clinicians’ decision to initiate invasive long-term ventilation,” was published in the European Journal of Paediatric Neurology.

In the survey, clinicians were presented with a series of vignettes for patients in different situations. For example, one vignette might describe a 16-year-old patient with substantial problems breathing but lots of familial support, while another might describe a 13-year-old with better respiratory function but less support from family. For each vignette, the clinicians rated how likely they would be to recommend invasive ventilation.

A total of 504 vignettes were rated by 249 clinicians (mostly doctors, but also some nurses and other providers). Using the survey findings, the researchers constructed statistical models looking for factors that were most predictive of the decision to recommend invasive ventilation.

Parents agreeing to invasive ventilation as a right choice was decisive

By a substantial margin, the most influential factor was whether a patient’s parents were in agreement that invasive ventilation is the right choice, results showed. Irrespective of all other factors, clinicians were nearly 13 times more likely to recommend going through with invasive ventilation when the parents were on board.

The next most influential factor was familial support: Clinicians were more than twice as likely to recommend invasive ventilation for patients with a strong family network.

Distance to specialty care also was influential. Specifically, clinicians were more likely to recommend invasive ventilation for patients who live within an hour of a specialty center, compared with patients living more than three hours away.

These factors “all point to clinician awareness and acknowledgement of the perceived challenges likely to be experienced by the family post-initiation, or as a result of their decision not to initiate,” the researchers wrote. They noted that open-ended comments submitted as part of the survey also reflected this idea, as clinicians consistently emphasized the importance of clear and open communication with families.

But the scientists also noted that the views expressed by adolescents with Rett syndrome were generally given much less weight than the views of their parents. This is likely because Rett patients frequently have trouble communicating, so it’s often hard for them to meaningfully engage in these complex discussions.

Nonetheless, “the perspective of the adolescent is extremely important,” the scientists stressed. They urged clinicians and families to include patients in these talks where feasible, noting that “communication strategies used with adolescents living with [Rett] continue to improve and there is a push for more inclusive practices where possible.”

This study was funded by the European Research Council as part of a larger research program called TechChild.